摘要:

AbstractPlasma level of beta‐thromboglobulin (BTG) and platelet factor 4 (PF4), two platelet‐specific proteins, were measured in 20 patients with cancer. None of the patients had evidence of thromboembolism or impaired renal function, and none was taking drugs known to interfere with platelet function. In ten patients who were in complete remission or whose disease was thought to be inactive, the levels of both proteins were essentially within normal limits. The other ten patients had active or progressive symptomatic disease. BTG level was elevated in all but one patient, and PF4 was raised in two patients. These results suggest that in patients with clinically active cancer, a state of ongoing in vivo platelet activation may be present, a phenomenon which may contribute to the known increased occurrence of thromboembolism in patients with malignancy. The intravenous administration of various cytotoxic drugs used in cancer chemotherapy produced no immediate measurable changes in BTG and PF4 le

ISSN:0361-8609    

DOI:10.1002/ajh.2830140202

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1983

数据来源: WILEY

摘要:

AbstractA rare familial case of hereditary stomatocytosis with hemolytic anemia, increased auto‐hemolysis, increased osmotic fragility, and shortened erythrocyte survival is described. The erythrocytes were abnormally permeable to sodium and potassium. In addition, “Na‐K pump” rate of the red blood cells was increased, while Na+‐K−‐ATPase, Mg2+‐ATPase and Mg2+‐Ca2+‐ATPase activities were within normal limits. Splenectomy induced marked im

ISSN:0361-8609    

DOI:10.1002/ajh.2830140203

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1983

数据来源: WILEY

摘要:

AbstractStructural studies and quantitative analyses were conducted on the hemoglobin of 55 newborn babies. Seven α chain variants (G‐Philadelphia, Montgomery, Inkster, I‐Philadelphia, Matsue‐Oki, Winnipeg, and O‐Indonesia) were present in 26 heterozygous new‐borns (17 black, eight Caucasian, and one Indonesian). The relative amount of the αxcontaining abnormal Hb F of the Hb G‐Philadelphia and Hb Winnipeg babies was less than observed in heterozygous adults, which may indicate a decreased rate of assembly of the αx‐γ dimer over that of the αx‐β dimer. Of the 29 newborns with γ chain variants, 16 were Caucasian babies; of these 15 had a HbAγ F‐Hull heterozygosity and one a HbGγF‐Marietta heterozygosity. Six black babies were heterozygous for HbAγF‐Texas‐I and six for HbGγF‐Port Royal. One Japanese baby had a heterozygosity forAγF‐Iwata and a second was heterozygous forATF‐Yamaguchi. Quantitative analyses of the isolated normal Hb F0as well as an evaluation of the relative amounts of the Hb Fxin the red cell lysates gave data useful for a speculation of the genetic condition in each of these babies. It was concluded that the babies with the Hbs F‐Texas‐I, F‐Iwata, F‐Hull, and F‐Marietta were simple heterozygotes with either theGγ,Aγ/Gγ,AγNor theGγ,Aγ/Gγx,Aγ genic arrangement. The babies with Hb F‐Port Royal had aGγ,GγxGγ,Aγ arrangement, which may result from a (to be determined) gene conversion. The newborn baby with Hb F‐Yamaguchi has theGγ,Aγ/Aγ/TN.—. genic arrangement, suggesting the presence of three distinctly different γ chain genes of which one, theAγT‐Xgene, produces anAγ chain (with threonyl at position γ75and an Asn at position γ80) at a level usually seen forGγ rather thanAγ chains. These studies we

ISSN:0361-8609    

DOI:10.1002/ajh.2830140204

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1983

数据来源: WILEY

摘要:

AbstractThe γ chain compositions of the fetal hemoglobins of 2453 newborn babies from East Asian countries (1350 babies), from Italy, Yugoslavia, Bulgaria, and Georgia (417 Caucasian babies), and 686 black babies from Georgia were determined by high pressure liquid chromatography. Unusual results for a limited number of babies were confirmed by chemical analyses, and were evaluated further by family studies.Statistical analyses indicated high gene frequencies for theAγTchain in Italian (f = 0.237), Yugoslavian and Bulgarian (f = 0.238), and white Georgia babies (f = 0.224), a lower frequency in Japan (f = 0.178), and India (f = 0.173), and particularly in mainland China (f = 0.079). TheAγTgene frequency in normal (AA) Black babies was 0.102. When a βSor βCmutation was also present this frequency was greatly decreased, particularly in babies with the AC condition (f = 0.036). These results suggest the near absence of theAγTmutation on the chromosome also carrying the βCdeterminant.Most babies had the expectedGγ values which vary between 60 and 80%, but several (mainly black) babies had higher values (between 80 and 90%), while one normal black baby had aGγ value of (nearly) 100%. This condition may be a form ofAγ+1‐thalassemia and has been discussed in detail elsewhere (Blood 58:491‐500, 1981). Thirty‐five clinically normal (mainly Chinese, Indian, and Japanese) babies hadGγ values of about 40%. Twenty‐six babies hadAγ1values of about 60%, while the remaining nine babies hadAγTandAγIchains in a ratio of either 1 to 2 or 1 to 1. Two additional newborns did not produce anyGγ chains, but had onlyAγIchains orAγIchains. Family studies failed to indicate a specific hematological abnormality. These unusual ratios between theGγ and (eitherAγIorAγT) chains have led to speculations regarding possible genetic abnormaliti

ISSN:0361-8609    

DOI:10.1002/ajh.2830140205

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1983

数据来源: WILEY

摘要:

AbstractThe in vitro sensitivity of circulating progenitor cells (CFU‐C) of 20 patients in blast crisis of chronic granulocytic leukemia (CGL) to vincristine was correlated with the clinical response to vincristine in vivo. Eleven patients who achieved either a good or partial clinical response displayed a reduction in the number of colonies or clusters formed by their peripheral blood leukocytes in a double layer agar culture assay following incubation with vincristine. The CFU‐C of five of six patients who failed to respond clinically to vincristine and prednisone were not suppressed following incubation with up to 12 μM vincristine. Three additional patients were not evaluable due to early post‐treatment deaths. In vitro assay of the effects of vincristine on CFU‐C appears to have predictive value for in vivo response in blast crisi

ISSN:0361-8609    

DOI:10.1002/ajh.2830140206

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1983

数据来源: WILEY

摘要:

AbstractMonocytes with binding capacity to sheep red blood cells (SRBC) are described in a case of acute undifferentiated leukemia with blast cells showing no cytochemical or immunological markers. The monocyte nature of the rosetting cells was confirmed by intense reactions of naphthol AS esterase and α‐naphthyl esterase and sometimes by peroxidase positivi

ISSN:0361-8609    

DOI:10.1002/ajh.2830140207

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1983

数据来源: WILEY

摘要:

AbstractTwo kindreds of phosphofructokinase (PFK) deficiency associated with congenital nonspherocytic hemolytic anemia and mild myopathy were found in Japan. Both probands had jaundice, gallstones, and slight to moderate degree of exercise intolerance. They showed decreased level of red cell PFK activity and no increase of blood lactate in forearm ischemic exercise test. We studied these probands' red cell PFKs by partial purification and condensation. Muscle type isozyme of PFK in both cases was not demonstrable in starch gel electrophoresis and DEAE‐Sephadex chromatography. The clinical symptoms are considered to be due to a defect of muscle type isozym

ISSN:0361-8609    

DOI:10.1002/ajh.2830140208

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1983

数据来源: WILEY

摘要:

AbstractThis report describes a patient with immunoblastic sarcoma and autoimmune hemolytic anemia with reticulocytopenia and red cell hypoplasia. Both IgG and IgM red cell autoantibodies were present in the patient's serum, and both antibodies had the uncommon specificity of anti‐IT. This report appears to be the first to describe IgG auto anti‐ITand autoimmune hemolytic anemia in a patient who did not have Hodgkin's disease; the laboratory findings and clinical course are described. The reticulocytopenia and red cell hypoplasia may have been immunologically mediated. The patient responded to plasma exchange, corticosteroids, and chemother

ISSN:0361-8609    

DOI:10.1002/ajh.2830140209

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1983

数据来源: WILEY

摘要:

AbstractA 32‐year‐old white male with agnogenic myeloid metaplasia developed pericardial tamponade shortly after elective splenectomy. Tamponade was relieved by pericardiocentesis but recurred, necessitating partial pericardiectomy. Following surgery and a course of radiation therapy to the heart, there was no further significant reaccumulation of fluid. Pathologic evaluation demonstrated foci of extramedullary hematopoiesis in the pericardium. To our knowledge, pericardial tamponade has not previously been reported as a biopsy‐proven complication of myeloid metap

ISSN:0361-8609    

DOI:10.1002/ajh.2830140210

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1983

数据来源: WILEY

摘要:

AbstractA patient with hairy cell leukemia experienced two episodes of an unusual neurologic disturbance associated with vaccination. The association with polyvalent pneumococcal vaccine has not previously been noted.

ISSN:0361-8609    

DOI:10.1002/ajh.2830140211

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1983

数据来源: WILEY